J Kartal TR 2016;27(2):142-144
doi: 10.5505/jkartaltr.2015.015428
CASE REPORT
OLGU SUNUMU
Supratentorial Hemangiopericytoma: Case Report
Supratentorial Hemanjiyoperisitom: Olgu Sunumu
Correspondence: Dr. Necati Tatarlı.
Şemsi Denizer Cad. E-5 Karayolu Cevizli Mevkii, 34890 Kartal, İstanbul
Phone: 0216 - 441 39 00 / 1351
Received: 20.10.2014 Accepted: 23.10.2014 Online date: 10.06.2016
e-mail: necatitatarli@gmail.com
Introduction
Hemangiopericytoma (HPC) accounts for less than 2% of soft tissue sarcomas, and it is a vascular tumor more frequently seen in retroperitoneum, hips, and thighs of children.
[1–3]Intracranial HPC is rare and can have clinical features resembling meningioma.
[4]Intra-
cranial HPC accounts for 0.4% of primary central ner- vous system (CNS) tumors; it is generally localized in supratentorial region.
[2,5]The World Health Organiza- tion (WHO) considers it a grade 2 tumor. It is known as mesenchymal tumor with malignant potential.
[6]Necati TATARLI,
1Yusuf Emrah GERGİN,
1Selçuk ÖZDOĞAN,
1Dilek YAVUZER,
2Mehmet TİRYAKİ,
1Tufan HİÇDÖNMEZ
1Özet
Hemanjiyoperisitomlar, herhangi bir yerindeki kapiller peri- sit hücrelerinden kaynaklanırlar. Nadir görülen vasküler tü- mörler olup, en çok retroperiton, kalça ve uylukta izlenirler.
Çoğunlukla supratentoriyal olarak görülen intrakranial he- manjiyoperisitomların, klinik ve radyolojik bulgularla menin- giomlardan ayrılması güçtür. Tüm primer santral sinir sistemi tümörlerinin %0.4’üdürler. Dünya Sağlık Örgütü (DSÖ) tara- fından derece II olarak kabul edilmektedirler. Malign potan- siyelli mezenkimal tümör olarak bilinirler. Meningeal heman- jiyoperisitomlar çok nadir görülen tümörler olup, genellikle supratentorial olarak yerleşirler. Ayırıcı tanıda meningiyoma dikkat etmek gerekir. Kesin tanı, cerrahi sonrası histopatolojik çalışma ile konmaktadır. Bu yazıda, 37 yaşındaki erkek hasta- da meningeal hemanjiyoperisitom olgusu sunuldu, klinik ve patolojik özellikleri ele alındı. Literatürde nadir olması nede- niyle bu olgu sunuldu.
Anahtar sözcükler: Beyin tümörü; hemanjiyoperisitom; meningi- yom; supratentorial.
Summary
Hemangiopericytoma (HPC) can originate in capillary peri- cytes of any part of the body. It is a rare vascular tumor, most- ly seen in retroperitoneal space, hips, and thighs. Based on clinical and radiological findings, it is difficult to distinguish supratentorial intracranial HPC from meningiomas, Intracra- nial HPC accounts for 0.4% of all primary central nervous system (CNS) tumors. The World Health Organization (WHO) classified it as grade 2 tumor, and mesenchymal tumors are characterized as having variable malignant potential. Men- ingeal HPC is an extremely rare tumor, usually found in su- pratentorial location. Meningioma should be considered in differential diagnosis. Definitive diagnosis is made with his- topathological analysis after surgery. In the present report, clinical and pathological features of a 37-year-old male pa- tient who presented with meningeal HPC are described. This case is presented because of the scarcity of such instances in the literature.
Keywords: Brain tumor; hemangiopericytoma; meningioma;
supratentorial.
1Department of Neurosurgery, Kartal Dr. Lütfi Kırdar Training and Research Hospital, İstanbul, Turkey
2Department of Pathology, Kartal Dr. Lütfi Kırdar Training and Research Hospital, İstanbul, Turkey
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Case Report
A 37-year-old male patient presented at clinic with complaints of progressive headache and generalized tonic-clonic convulsions. Neurological examination was unremarkable. On cranial magnetic resonance imaging (MRI), extracranial mass lesion with periph- eral intense edema measuring 42x28x35 mm that dif- fusely retained contrast media was seen localized in left frontoparietal junction (Figure 1a, b). Surgery was performed with initial diagnosis of meningioma Mass was totally excised with its dura. Duraplasty was per- formed using galea graft. No intra- or postoperative complications developed.
Histopathological examination revealed tumoral hy- pervascular mass consisting of randomly scattered monotonous cells with round-oval nuclei and thin wall which appear as clefts. Tumoral cells had fine chromatin structure and small nuclei with indistinct cytoplasma contours. No prominent cytological atypia or necrosis was found. A 4/10 BBA mitosis was detected. On histochemical examination, patchy ar- eas stained with CD34 antibody and B cell lympho- ma/leukemia gene-2 (Bcl-2) were found, while epi- thelial membrane antigen (EMA), and S–100 protein positivity were not observed. Based on these find- ings, tumor was diagnosed as HPC grade 2 (Figure 2a-c).
Patient later underwent radiotherapy, and in postop- erative third year had experienced no disease recur- rence.
Discussion
HPC was first defined by Stout and Murray in 1942, and it has been suggested that HPC may derive from Zimmermann pericytes.
[7]Meningeal HPC was first
(a) (b)
Figure 1. (a) On preoperative T1- weighted contrast-en- hanced magnetic resonance examination per- formed on axial plane, an extraaxial mass measur- ing 42x28x35 mm with a diffuse peripheral edema localized on the left frontoparietal junction is seen (b) T1-weighted contrast-enhanced magnetic resonance examination performed on axial plane demonstrates an extraaxial mass lesion.
Figure 2. (a) Tumor consists of monotonous cells with hypervascular thin wall, and round-oval nuclei (H-E, x200). (b) Patchy areas of CD34 positivity in tumor cells (immunohistochemistry, x100). (c) Tu- mor calls were not stained with EMA dye (immu- nohistochemistry x200).
(a)
(b)
(c)
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described by Begg and Garret in 1954.
[8–10]Meningeal HPCs are generally seen in young peo- ple, with equal distribution between genders.
[11]Symptoms vary with anatomical location of tumors.
Frequently, no calcification is present. Because of adjacency to dura and small size, it resembles menin- gioma; however, meningioma and HPC are complete- ly different pathologies.
HPC grows gradually and leads a painless course because of its soft tissue involvement.
[2]It is usually devoid of any capsule, however encapsulated cases have been also reported.
[3,4,7]Microscopic evaluation reveals diffuse reticulin fibers and abundant num- ber of large, well-circumscribed cells with little cyto- plasm.
[12]In cases with meningeal HPC, generally, surgical exci- sion is the preferred method for both diagnostic and therapeutic purposes. Some cases have responded satisfactorily to postoperative radiotherapy. However, without surgery, pericytoma usually responds poorly to chemotherapy and radiotherapy.
[1]Postoperative radiotherapy may be desirable due to its survival pro- longation effects. Pericytoma frequently metastasizes to liver, lungs, and musculoskeletal system.
[1]Menin- geal HPC is a very rarely seen tumor, generally local- ized in supratentorial region. Meningioma should be considered in differential diagnosis. Definitive di- agnosis is based on postoperative histopathological analyses.
Conflict of interest None declared.
References
1. Seo I, Kim YS, Kim HS, Kim JH, Lee MK. Hemangiopericy- J Kartal TR 2016;27(2):142-144 doi: 10.5505/jkartaltr.2015.015428
